A
REPORT ON A SURVEY OF LATE EMERGING MANIFESTATIONS OF CONGENITAL RUBELLA
SYNDROME
By
Nancy O’Donnell, HKNC, Coordinator of Special Projects
The Helen Keller National Center for
Deaf-Blind Youths and Adults (HKNC), headquartered in Sands Point, NY, is the
only national agency to provide comprehensive vocational and rehabilitation
training exclusively to youth and adults who are deaf-blind. There are many
causes of deaf-blindness, but one group that has drawn our attention are those
who are deaf-blind from congenital rubella syndrome.
Until rubella was declared “eliminated” in
the
A review of the literature in the late
1980s revealed reports of additional health concerns developing in individuals
with CRS in Australia. This prompted HKNC to conduct a survey in 1989-1990 to
determine whether there were simliar health concerns in the U.S. population of
those who were deaf-blind from CRS. The results of that survey confirmed a
higher than expected incidence of glaucoma, diabetes, thyroid conditions,
esophageal problems and a degenerative process effecting physical and mental
abilities. Individuals with CRS, their family members and service providers all
indicated a desire for more information about this topic. Due to a scarcity of
research on this topic and the lack of a forum through which information could
be shared, HKNC established a listserv to facilitate discussions on rubella and
CRS among interested parties. This listserv has been in existence since 2000
and continues to provide a virtual community of information and support
internationally. Individuals from countries such as
1) The HKNC website maintains a list of
articles and resources on rubella
http://www.hknc.org/Rubella.htm
2) Through Deafblind International, we maintain a Rubella Network. For more
information, go to
http://www.deafblindinternational.org/standard/network_rubella.html
3) DB-LINK, the National Clearinghouse for Information on Deaf-Blindness, has
an extensive bibliography of articles on CRS, with some articles available in
full text on the website. Go to http://www.nationaldb.org/ISSelectedTopics.php?topicCatID=29
We continue to hear about late onset medical conditions developing in those
with CRS. Unfortunately, there has been little medical research conducted to clinically
determine a definitive link between congenital rubella syndrome and the various
medical conditions reported. Anecdotal reports of medical and emotional issues
surely convince us of the need for further clinical investigation. Families and
individuals living with CRS deserve answers to their concerns so that they can
manage their lives in a healthy and proactive way. We implore the medical
research community to renew their interest in, and commitment to, finding
answers about CRS.
As always, we appreciate your feedback. We
may not have all the answers, but we will continue to seek them.
STATEMENT OF THE
PROBLEM
This report will discuss information on
individuals with deaf-blindness whose parents/caretakers responded to a survey
regarding late emerging manifestations of Congenital Rubella Syndrome (CRS).
The survey was conducted in response to concerns expressed by parents and
service providers who had observed dramatic changes in behavior and/or medical
conditions in their adult children/clients with CRS. There has been very little
recent information written about this phenomena, although late onset medical
problems have been documented, particularly in the Australian Rubella
population bom in the 1940s. An
excellent resource for parents and professionals about CRS is the November 1980
edition of "American Annals of the Deaf," Volume 125, No. 8. The
entire volume is dedicated to the topic of rubella. Although the information is not current, it
provides a comprehensive overview of the population and the challenges they
face. Other resource articles are listed at the end of this report, in the
bibliography.
PROCEDURE
The survey was conducted in two phases. In Phase 1, which took
place from November 1989 to April 1990, an interviewer contacted
parents/caretakers by phone, asked questions and filled out a survey form while
talking with the parents. In Phase II,
June 1990 to 1991, a large scale mailing was conducted across the country. The mailing list was composed of names
received through parent organizations, rehabilitation agencies, the Helen
Keller network of Affiliated Agencies, HKNC Regional Representatives and
through word of mouth. Information was
requested on each person's:
§ Residential Placement
§ Employment
§ Hearing and vision
§ Communication methodology and
opportunities to communicate
§ Increase or decrease in negative
behaviors
§ Changes in lifestyle
§ Medical conditions and age of onset
Most of the questions were in a yes/no format or multiple
choice with space for comments.
Our goal was to gather information on individuals 16 years
of age and older who were reported to be deaf and blind as a result of CRS.
During Phase I of our effort, 39 interviews representing 19 states were
completed by telephone. Much of the information received during these
interviews was anecdotal in nature.
Since Phase II, we have received and analyzed 88 additional
responses representing another 14 states. The percentages reported herein are
based on those 88 responses. However, the
impressions, quotations and interpretation of the data also reflect the
wealth of information gathered from the initial 39 interviews.
The following report is divided into two main sections. Section I provides a general overview of the
respondents. Section II focuses on
medical information.
1.
GENERAL OVERVIEW OF RESPONDENTS
Fifty-eight percent of the respondents were male, forty-two
percent were female. The largest percentage of respondents (32 %) fell into the
25-26 year old category, reflecting individuals who were bom as a result of the
rubella epidemic of the 1960s.
A. Residential Profile
· 41 % were reported to live with
parents/family
· Almost 20% were in some type of group home
· I I % live in MH/MR facilities
(institutions, state hospitals)
· 9% were in foster care
· 9 % were in an intermediate care facility
B. Employment Profile
· Approximately 32% work in a sheltered
workshop or work activity center
* 27% are students
· 20% are in a training facility, including
employment training
* 2 % are competitively employed
· 3% are engaged in Supported Employment
·
Hearing & Vision
·
Almost
50% reported little or no useable vision, while 30% have significant problems
·
Approximately
90% were considered to have no functional hearing
·
45%
fell into the category of profoundly deaf with no vision and another 28% were
profoundly deaf with significant vision problems
D.
Communication
The following percentages indicate a yes/no response to a
particular method of communication. It does not rate the capability of the
individual in each category. Respondents
were allowed to check as many methods as applied.
Receptive
Methods
·
Out
of a list of ten possible methods for receiving communication, sign language
was the most frequently indicated (83%).
·
Gestures
and prompting were checked by 60% of the respondents.
·
One
fourth of the cases were reported to understand fingerspelling.
·
5
individuals could read braille (6%).
·
3
individuals were described as having no receptive method of communication (3%).
Expressive
Methods
·
Sign
language was the most common method of expressive communication (70%).
·
55%
are reported to use gestures.
·
11%
use speech, either alone or in combination with another method.
·
6
cases reported no method of expressive communication (7%).
Opportunities to Communicate
·
16%
of the respondents answered "no" when asked if there was someone in
the deafblind person's situation who could communicate effectively with that person.
·
23%
answered "no" when asked the same question in response to their work
placement/day program.
E. Behaviors
Respondents were asked to indicate
which out of 9 behaviors were currently demonstrated by the deaf-blind person
and whether the frequency of these behaviors had increased, decreased or
remained the same over the past year.
·
Self-stimulation
and vocalization were the most commonly occurring "behavior
problems," often reported every day. Approximately 60 cases (68%) reported
that this behavior decreased over the past year.
·
Disruptive
activities were the next most frequently noted behaviors. Ten individuals (11%)
were reported to do this every day. Forty percent (40 %) are disruptive only
occasionally. Approximately one-third of the respondents are not disruptive at
all.
·
Tantrums/outbursts
were noted at least once a week in 35 % of the cases. This behavior occurred
occasionally in one-third of the individuals. Almost 20% did not demonstrate
this behavior at all. The frequency of this behavior was noted to have
decreased or remained the same in 60 cases (68%) over the past year.
·
Self-injurious
behavior was reported to occur at least once a week in 35% of the cases. 40 %
did not demonstrate this behavior. About 40 cases (45 %) indicated a decrease
in this behavior since the past year.
·
The
most serious demonstration of behavior problems (Damages Property and Assaults
Others) were reported to occur in 11 cases (12.5%) at least once/week.
Thirty-four (34 %) were assaultive occasionally; 28 % damaged property
occasionally. Approximately 50% did not demonstrate either behavior.
In
general, there was a definite trend in all behaviors decreasing or remaining
the same over the past year.
F. Lifestyle Changes
Individuals with CRS are frequently
described as having difficulty accepting changes in their
routine/environment.Information was requested on the number and types of
changes that occurred during the past year.
The results were as follows:
·
27% - change in health
·
26%
- change in living arrangement
·
43%
- change in family/roommate/staff (residential)
·
33%
- change in work
·
36%
- change in staff/fellow employees
·
18% - change in transportation
Obviously, this is a group that
experienced significant changes in their environment.Many were involved in
several changes over the past year. What effect did these changes have on their
behaviors, their communication opportunities and their general quality of life?
How can we plan for more consistency, particularly in staffing patterns?
II. MEDICAL CONDITIONS
I would like to give special thanks to Barbara Fedun whose
knowledge, patience and expertise were cnitical in preparing this section.
This section is divided into seven categories according to
problems noted in the following areas:
A) Auditory Disorders
B) Ocular Damage
C) Cardiac Problems
D) Endocrine System (Diabetes and Thyroid)
F) Degenerative Conditions
G) Esophageal/Gastrointestinal Symptoms
H)
Miscellaneous
Each category will be introduced by
information cited in previous articles or studies. This will be followed by our own findings,
including comments, questions or impressions from parents who participated in
the survey.
A. AUDITORY
DISORDERS
“Long Term follow-up of these children
indicates that almost all eventually show signs of some neurologic damage, the
most common being sensorineural hearing loss, which approached 93% at seven to
nine years of age.” (2)
“The most common handicap associated
with rubella is hearing loss which affects about 73% of the cases. The losses
are generally bilateral and sensorineural.” (1)
“…The hearing defect may become
progressively worse after the first year of life. There have also been cases in
which children with CRS, for whom audiograms nd speech patterns are normal
suddently developo mild-to-profound sensorineural hearing loss. The latest age
at which this occurrence has been well documented is ten years.” (4)
Ninety-two percent of our respondents
in Phase II indicated that their child had little or no hearing at the time of
the survey. One case of late onset hearing loss was reported at age 10. Another mother mentioned that her daughter
seems to not hear as well 'as the years go by." In Phase I, however,
several parents reported a decrease in their child's hearing. One case involved
a female, age 20, who also developed glaucoma at age 16 and hirsutism (increase
in facial hair, body hair) accompanied by an increase in weight and breast
size. Another case involved a male, age 25, who also was experiencing many
seizures, had developed glaucoma at age 20, had a detached retina repaired at
age 24 and had demonstrated "alot" of head banging. A third case
involved a young man, age 24, whose mother has noticed a decrease in hearing
over the past five years. This individual also has glaucoma.
An interesting case surfaced, however,
during the telephone survey I will leave it to you to come to your own
conclusions:
One mother described a son with some
useable vision and no apparent hearing. At age 12, he lost his remaining
vision. Soon after this, she noticed that his hearing seemed to "kick
in." He began responding to environmental cues and directions. At the time
of this survey, he was 23 years old and his primary means of receiving
communication is aurally. How does the mother explain this unusual development?
She related this to information she had learned from a seminar she attended on
autism years ago. It was explained that many autistic children experience
"sensory overload" there is too much information coming in through
their eyes and ears which they can't process so the children just shut these
systems down. Teachers were reported to have more success if they focused just
on visual input or auditory, not both. Perhaps in her son's case, she
theorized, he had experienced the same type of overload. When he later lost his
vision, the overload was reduced and perhaps he was then able to "tune
in" to his hearing. Interesting food for thought.
B. OCULAR DAMAGE
Glaucoma with late onset was reported by Boger in 13
patients with CRS. The diagnosis was first made when the patients were between
2 and 22 years of age. In 11 cases, the eyes were microphalmic, 2 had eyes of
normal size. All had cataractous lenses either removed surgically or absorbed
spontaneously. (4)
In one study, almost 10% of patients with prior ocular
damage developed additional forms of eye damage as a delayed manifestation.
Cataracts occur in 25% of those infected between 25-93 days gestation.(4)
Cooper stated that 50% of the time, cataracts are unilateral
and associated with microphthalmia. In 1969, he reported that while he had seen
no patients with both cataracts and glaucoma, they do not appear to be mutually
exclusive in the same eye. (This is not to be confused with the glaucoma that
is secondary to a poor outcome of cataract surgery.) (10)
Cataracts
Sixty-six percent (66%) of our
respondents reported having cataracts, with 57% indicating that these were
congenital. Two cases of cataracts were identified at age 7, one case at age 16
and one at age 18. Several parents wanted to make sure that we include an alert
to other parents as to the increased risk of their children developing- glaucoma as a result of
cataract surgery. Late onset glaucoma has also occurred in children who never had
cataracts and in those whose unilateral cataract was never operated on. Please
be aware of this and have your sons and daughters tested for glaucoma on a
regular basis!
Detached Retina
There were 12 cases of detached retina (13%). For those who reported an age of onset, there
was one case diagnosed at each of the following ages: 2, 8, 10, 12, 16, 17, 18,
19, and 39.
I could not find any article which documented the incidence
of detached retinas in individuals with CRS or in the general population.
However consider that 35 % of our cases were reported to engage in
self-injurious behavior at least once a week. Some of this behavior (head
banging, eye poking) could undoubtedly contribute to retinal detachment.
Glaucoma
Thirty percent (30%) of our respondents were reported to
have glaucoma. This rate of occurrence is very hight, when compared to an
incidence of 0.5% in the general population. Our sruvey also asked whether
particular medical conditions had been tested for in the past year. Only 32%
responded that they had been tested for glaucoma in the past year. We strongly
recommend that glaucoma testing becomes part of the annual physical
examination. This precaution should be applied as well to CRS adults who are deaf with no previous
vision problems, as they have also been found to be developing glaucoma at much
higher rates than the general population. (12) Glaucoma prevention is critical
in order to try to maintain whatever vision an individual has, particularly in
light of additional handicapping conditions.
We have had at least one report of glaucoma that is
difficult to control by medication. One
parent described a 22-year old daughter (who had had nine eye operations since
birth) whose glaucoma was not responding to treatment.
C. CARDIAC PROBLEMS
_____________________________________________________________________________________
Several studies cited
the incidence of heart problems in the CRS population:
“35-76% of rubella youth have heart disorders.
Patent Ductus occurs in up to 58%; Ventricular Septal Defect occurs in up to
18%.” (1)
“Hypertension can appear later on due to renal artery or
aortic stenosis and may be associated with further occlusions of damaged
vessels…these vascular lesions are potential causes of coronary, cerebral and
peripheral vascular disease in adults.” (4)
“Heart defects occur in 30% of children
infected between 25 to 93 days of gestation.” (2)
“van Dijk, citing Menser, Dodds and Harley, 1967, states
that of the 50 high functioning persons born with CRS in 1940, twenty-five
years later, three were found with systemic arterial hypertension.” (9)
________________________________________________________________________________________
Fifty-two percent (52%) of our respondents indicated the
presence of a heart condition. Patent Ductus, Atrial or Ventricular Septal
Defects and Pulmonary Stenosis were the most common. In many situations, the condition was
coffected surgically in infancy. Twenty-eight out of 88 participants (32 %) answered
affirmatively regarding whether or not cardiac testing has been done in the
past year.
Parents did not have alot of concerns about heart problems
until August of 1990, when it was learned that one of our 39 initial
participants passed away due to congestive heart failure. In a low incidence
population, such as ours, one case like this could have serious implications.
However, it was later determined that this 26 year old male had had structural
repair to his heart in infancy. Although
devastating to the family, this was not a medically unexpected outcome for
someone with a long history of complicated cardiac disease.
Another case which created interest was a female, now 28
years old, who was part of an ongoing study on congenital heart defects
conducted by
We present these two isolated cases as a portrayal of the
wide variety of medical conditions and challenges being faced by this
population. We are continuing to review
this information to try to identify trends and further areas for study.
D. ENDOCRINE SYSTEMS
Diabetes
____________________________________________________________________________________________________
“Research in programs at the
“ The most frequent delayed
manifestation of CRS is diabetes mellitus. Studies reported by Menser, et al,
of patients from the rubella epidemic in 1941 in
“A high incidence of overt diabetes
mellitus is seen among patients with…(CRS)…who reach adolescence (10%) and an
almost equal number of young adults with stigmata of CRS show abnormal glucose
tolerance responses.” (2)
O’Dea and Mayhall cite Rayfield, Kelly
and Yoon, (1986) “the incidence of diabetes…in association with CRS has been
reported to be between 12 and 20 percent.” (6)
Our survey indicated 5.7 % (5 out of 88) of our participants
are diabetic. The ages of onset were
reported as follows:
Age of Onset # of
Cases
2 1
5 1
14 1
24 1
Unknown 1
Although our percentage is lower than those indicated in
previous studies, it is approximately twenty times higher than the incidence
found in the general population. (12) We must also consider that the bulk of
the population in our survey were in the 25-26 year old age range. The
Australian study on diabetes included patients up to 35 years old. It will be
interesting to see what the next 10 years brings to us here in the States.
In terms of testing, 16 out of 88
respondents (18%) indicated that their son/daughter had been tested for
diabetes in the past year. We should keep in mind the symptoms of diabetes
include excessive thirst, frequent urination, rapid weight loss, nausea and
vomiting.
Please note that several parents also
mentioned that their child's blood sugars fluctuate a great deal. This has been observed by Fedun, as well, in
her ongoing study of the relationship of
diabetes and CRS. (12)
Thyroid
“Hypothyroidism, hyperthyroidism and
thyroiditis have all been reported as delayed manifestations of CRS…” Thyroid
dysfunction has been identified in 5% of patients with CRS in one study. (4)
“…thyroid disease has been relatively
rare…” (10)
“Once
again, infection followed by an autoimmune response has been indicated as a
possible cause of thyroid disease.” (6)
E.
DEGENERATIVE CONDITIONS
This section contains some of the most puzzling and
disturbing descriptions of changes occurring in individuals with CRS. The
following comments written by parents will give you a glimpse into the types of
degenerative symptoms some children are developing. The first case describes a
26 year old male.
'Over the past
four years, (our son) has gradually lost his ability to walk. In the past year,
he cannot stand by himself nor walk at all without totally leaning on another
.... (He) was always very hyper ... also very aggressive. He still butts his
head quite often and pinches if he is near us. We were recently told that (he)
is on a down-hill slide and will eventually lose all control, including
muscles, swallowing, etc...The doctor does not know another patient with (this)
condition. The doctor advised us to
remodel our house to accommodate a wheelchair. Do you know how long a time
between wheelchair and bed? "
Another parent described her 24 year
old daughter:
'... about six
years ago (she was 18) things first changed. She had one seizure She became
much more irritable, broke lots of windows, would actually swing at people
close to her, wasn't as willing to do things she had always loved and was
generally terrible. Her tantrums were frequent, violent and long-lasting. This
went on for 2 to 4 years to some degree. About 2 years ago she calmed down some
but in November all at once she became very unsteady on her feet. I thought she
may have had a stroke.Her gait is much like that of an'individual with cerebral
palsy. She looked 'drunk' all the time.
She had a great deal of trouble walking three feet without hanging onto
something. She appeared real confused over this change of events and began to
really depend on us to help her get from place to place. But the tantrum
stopped.
At this point we
had a CT Scan done, took her off her Phenobarb, took her to a heart specialist,
otorhinolaryngologist, neurologist and just about everyone else we could think
of – no one knows what's going on. Recently I requested an MRI. That did show a
large 'scrambled egg' appearing thing. The report ruled out a brain tumor,
mentioned in passing and rejected MS so now we know there is something in/on
her brain but don't know what it means. They also don't know if it is something
'new' or has always been there. How I wish we had been able to have tests like
this when she was a baby.
Right now she has
been “off balance” for a year and a half. She has adapted her once springy gait
to a wide stance, is sometimes amused, sometimes frustrated over her new
limitations (above I observe from behavior and facial expressions - she can't
tell me these things) and does not seem to have regressed any more during the
last year and a half.'
A third mother provided this account of
her daughter, now 26 years old:
'When (she) began
her weight gain in 1982, almost overnight after being so thin since birth, I
felt we had a problem. Along with this extreme weight gain, she began tantrums,
etc. Also (her) beautiful, smooth complexion turned to pimples and acne...(her
back was also like this). (Her) behavior became so aggressive, I could not
handle her (1986). With all the above, she developed facial and chin hairs, and
cleavage hairs. Hirsutism was diagnosed. Ever since 1982, there has been one
thing and then another. Diabetes -diagnosed 1988, losing control of (bowel) and
bladder (what appears to be aging faster on inside than outside), eyesight declining
due to congenital glaucoma, even though the medication is present, it doesn't
seem to be effective. On the other side of the coin, we have seen the most
miraculous advancement in (her) mental abilities.7his has been since 1988. It
is almost scary sometimes because she has these conversations with you that are
so 'adult' and completely normal. (She) talks with wisdom and great reasoning
abilities! "
During the course of this survey, I
have spoken to many parents and professional and listened to questions about
these degenerative processes. Often, the discussion would turn to a condition
called Progressive Rubella Panencephalitis (PRP). To gain additional knowledge
on the condition, I wrote to Dr. Jerry Wolinsky at the University of Texas
Health Science Center in
-------------------------------------------------------------------------------------------------------------------------------------
“Progressive rubella panencephalitis
(PRP) was recognized as a separate disease entity in 1975 when two groups
described patients with slowly progressive neurologic deterioratoins…(PRP) is a
rare disease with only about twelve known cases. Seven of these have been well
documented; five are with the known stigmata of (CRS) and two with postnatally
acquired rubella.” (2)
“A relatively stereotyped clinical
syndrome has been defined based on …ten cases. The syndrome is not
geographically restricted and, to date, appears only in males…Between the ages
of 8 and 19 years, neural deterioration becomes apparent. Deterioration in
school performance and behaviors are early symptoms, sometimes associated with
seizures. With time (there is) a progressive global demential…The gait becomes
clumsy early. Ataxia becomes prominent and progresses to involve all limbs…The
syndrome evolves slowly but relentlessly over several years.” (3)
“Intellectual function is dramatically
impaired…All known cases of PRP thus far have proven fatal.” (5)
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It must be emphasized that PRP is a
very rare disease which has only been well documented in 5 cases of individuals
with CRS. There are very specific and complex tests, as described in these
articles, that are necessary in order to come up with a diagnosis of PRP.
So, if not PRP, then what else could
explain the deterioration that is happening to some children? In their article, published in 1985, Sever,
South and Shaver surmised that “new neurologic abnormalities may occur that are
not followed by the relentless deterioration that is characteristic of PRP.
These abnormalities include behavior disorders and seizure disorders, but are
not associated with the ataxia of PRP... Such abnormalities probably represent
another delayed effect of earlier acute
rubella encephalitis." (4)
We will continue to follow these
phenomena and encourage any insights and information that the readers can
share.
F. ESOPHAGEAL/GASTROINTESTINAL
This category has been created in order
to highlight reports of gagging, and vomiting. These symptoms do not seem to
fit into any type of pattern which we could identify. They first surfaced
during conversations with several parents who participated in the telephone
survey. In two cases, it involved young men who also have cerebral palsy. The
young woman described in the section on Heart Problems also developed a problem
with gagging. Phase II of the survey brought us more examples of this behavior.
One parent described these symptoms in
her 19 year old daughter:
"7he past
year (especially 3-5 months) w have had some strange behaviors come and go with
(our daughter). Your late onset manifestations of CRS was very insightful as
(she) is definitely experiencing changes, whether physical or emotional is
anyone's guess. She has had some weight
loss, decrease in appetite with intermittent crying spells at times,when crying
she does a strange throat sound prior to this - then just sobs. During these times she gags violently as if
to vomit but doesn't. If anything it’s only mucous. She has had most exams done
- blood tests, urine - complete dental checks under anesthesia and we have
found no obvious physical ailment.”
This was written by the mother of a 24
year old daughter:
'The doctors are
unable to determine the cause of her recurring vomiting (every 3-9 weeks for
past six years). '
The Canadian survey verified this
finding in their population. (
G. MISCELLANEOUS
Many parents had
questions/theories/suggestions that they wanted us to pass on to other
parents. In some cases, we have been
able to include these in specific sections of the report. The remainder are presented here.
'Please let other parents know that
these children have smaller than average extremities. Their
feet are small. This
poses a problem for those who have put on extra weight. There could be pressure
on the ankle area and problems supporting all that body weight. '
'Medic Alert is creating a special registry for patients who
have had implants (such as pacemakers) in case there is a product
recall.Contact Medic Alert for more information.'
"In my (CRS) son's group of peers at school, the
siblings bom after the rubella child seemed to have more than their share of
their own problems. My daughter had a condition on her retina which looked like
a 'rubella' retina. Other children had
siblings with learning disabilities, vision or hearing problems. Is there any medical basis for this, or is it
just a coincidence?'
'I am glad you are
doing this survey ... I am am interested in psychological factors /symptoms
which seem to change - compulsive behawor, aggression, anxiety, "ticks, '
etc. What can we blame on rubella and
what can we blame on our child's 'gene pool?' Good Luck!'
"We are
interested to know any further developments with the late onset of CRS. Anything we can do to prevent these problems
from developing?'
"Are other
parents hawng as difficult a time getting medical insurance for their child as
I am? My son has been turned down
several times, even with the suggestion that they put a 'rider' on his eyes.
"Are these
children aging more quickly because of the rubella? Will that mean that they
have a shorter life expectancy? What are the implications for planning their
future?"
"My son has been experiencing
seizures since he was a young boy...not the commonly known types of seizure,
like gran mal' and 'petit mal. For example, have you ever heard of abdominal
seizures? When he was young, he would throw up and then fall asleep right after
that. After being put on seizure medication, this stopped.He later outgrew
them.At age 18, seizures started again. Recently they have become more
frequent. Many of the symptoms of his seizures could have been mistaken for
'bad behavior.” ( Author’s note: These seizures were eventually controlled through
medication.)
GENERAL CONCLUSIONS
By studying all of the data and listening to the parents who
voiced their concerns, an interesting picture is emerging of the adults with
CRS who are represented in our survey.
Almost a third of our respondents indicated no sign of late emerging
medical problems. The problems that were reported ranged in severity from
individuals who "only' developed glaucoma to one extreme case of a woman
who developed every late onset symptom on our list except diabetes. (Author’s note
– in 2000, this woman’s mother reported that her daughter developed diabetes as
well.) Researchers still do not
understand the mechanism which triggers the development of these conditions.
Three possibilities are autoimmune response, reactivation of the virus and
persistence of the virus. The probability of any one person developing a
particular condition is unknown as is a time frame in which these things might
occur.
We can expect that individuals with CRS
will be susceptible to the same ills that the general population develops. We
can only guess as to the effects of the various stresses on their lives, health
and behaviors. If we look at the survey
respondents as a whole, we see a severely involved group of young men and
women, many profoundly deaf and totally blind or with significant vision
problems, with limited communication systems, sometimes with limited
opportunity to communicate, They are undergoing, or have the potential to
experience, significant changes in their medical condition, yet are very
limited in their ways to tell us about
their symptoms. They are often subject
to changes in their living and/or working situation, yet are often unable to be
prepared for these changes because of their own limited communication or that
of the people around them. If we feel
any sense of urgency in relation to this population, it is, of course, in
regard to their medical status first. However, one simply cannot ignore the red
flags surfacing in the area of "communication." At least a third of
the parents with whom I spoke on the phone specifically mentioned their
concerns about the lack of communication instruction/intervention/opportunity
that their child had, especially those who are out of the educational system.
In looking at behaviors as communication,
our data depicts a higher frequency of negative behaviors in those who have the
least opportunity to communicate at work and at home. These individuals are not
"bad" ... they are frustrated, sad, confused, bored, angry, not
feeling well, etc., and they are trying to tell us this in the only way they
know how. We who are communicatively "intact" need to do a much
better job of figuring out what these individuals are trying to say and helping
them to say it in more appropriate and effective ways.
If you are looking for a good resource
book for yourself or for those who work with your son or daughter, I would like
to recomment two books: Enhancing Nonsymbolic Communication Interactions Among
Learners With Severe Disabilities by Ellen Siegel-Causey and Doug Guess. It is
an excellent introduction to understanding basic communciation attempts. It
gives concrete examples and suggestions on how to develop a communication
program, including guidelines and strategies. Remarkable Conversations: A guide
to developing meaningful communication with children and young adults who are
deafblind by Barbara Miles and Marianne Riggio is another excellent resource.
It is available through
As you can see, we don't have all
"the answers." It is obvious that there is a tremendous amount of
work to be done and the information gathered must be shared. If you have any comments or questions after reading
this report, new information you want to add to the survey, or if you have not
yet participated in the survey and wish to do so please write or call: Nancy
O'Donnell, HKNC,
BIBLIOGRAPHY
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